Abstract
Calcium-modulating cyclophilin ligand (Caml) is a ubiquitously expressed cytoplasmic protein that is involved in multiple signaling and developmental pathways. An observation in our laboratory of a protein–protein interaction between Caml and the cytoplasmic region of Cadherin23 led us to speculate that Caml might be important in the inner ear and play a role in the development and/or function of hair cells. To address this question, we generated a mouse line in which Caml expression was eliminated in Atoh1-expressing cells of the inner ear upon administration of tamoxifen. Tamoxifen was administered immediately after birth to neonates to assess the effect of loss of Caml in the inner ear during postnatal development. Hearing in treated animals was tested by auditory brain stem response (ABR) analysis and cochlear pathology was evaluated by light microscopy. Lack of Caml expression in the inner ear leads to severe loss of cochlear hair cells and complete deafness. Elucidating the role of Caml in the inner ear will aid our understanding of the molecular pathways important for auditory development and function.
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Acknowledgments
This work and EM's participation in the University of Missouri Veterinary Research Scholars Program were supported by University of Missouri Research Animal Diagnostic Laboratory research funds. ABR/EP recordings and cochlear histologic analysis were supported by R01 DC08321 (KKO) and P30 DC0046645 (R. Chole). We thank Dr. Beth Bauer for advice on aspects of the animal work and Howard Wilson for assistance with graphical design.
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Bryda, E.C., Johnson, N.T., Ohlemiller, K.K. et al. Conditional deletion of calcium-modulating cyclophilin ligand causes deafness in mice. Mamm Genome 23, 270–276 (2012). https://doi.org/10.1007/s00335-011-9381-z
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DOI: https://doi.org/10.1007/s00335-011-9381-z